ABSTRACT
Superficial morphea, a newly described variant of morphea, manifests clinically with hypopigmented or hyperpigmented patches that lack induration. The lesions show on biopsy dermal sclerosis in the superficial to mid-reticular dermis. So far, all cases reported in the literature were skin limited. In this report, we describe a 24-year-old female patient who developed at the age of 4 years plaques characteristic, both clinically and histologically, of superficial morphea. The patient developed later on psoriasis vulgaris. Although the association of scleroderma and psoriasis is rarely reported in the literature, most reports describe a progressive systemic course of scleroderma whenever the two diseases co-exist. This is the first report to describe superficial morphea in association with psoriasis and to provide a 20-year follow-up period during which the superficial morphea remained relatively stable with no evidence of systemic involvement.
Subject(s)
Psoriasis/complications , Psoriasis/pathology , Scleroderma, Localized/complications , Scleroderma, Localized/pathology , Adult , Age of Onset , Child, Preschool , Female , Follow-Up Studies , HumansABSTRACT
BACKGROUND: Macular arteritis (MA) is a term coined for a cutaneous vasculitis that manifests as multiple macules that favor the lower extremities and that reveal lymphocytic inflammation of an artery in the deep dermis and subcutaneous fat. METHODS: We describe the demographic, clinical, histologic, and laboratory findings in three new cases and review seven previously reported cases of MA, and discuss the nosology of MA. RESULTS: MA affects predominantly females (70% or 7/10) and individuals of African-American race (50%). Eight patients had hyperpigmented patches, one had hypopigmented macules, and one had erythematous macules. The lower extremities were involved in 100% of cases, followed by the upper extremities (44%). Light microscopic changes are those of a medium-vessel lymphocytic arteritis. Investigations for possible causes are non-revealing. CONCLUSION: We conclude that MA is a primary idiopathic cutaneous lymphocytic arteritis that is limited to the skin.
Subject(s)
Arteritis/immunology , Arteritis/pathology , Lymphocytes/immunology , Skin Diseases/pathology , Adult , Female , Humans , Hyperpigmentation/pathology , Middle Aged , Skin Diseases/immunologySubject(s)
Blister/pathology , Chickenpox/pathology , Biopsy, Needle , Blister/virology , Chickenpox/diagnosis , Child , Humans , Immunohistochemistry , Male , Prognosis , Rare Diseases , Risk AssessmentABSTRACT
BACKGROUND: Idiopathic atrophoderma of Pasini and Pierini (IAPP) usually manifests as one or multiple depressed and hyperpigmented patches, with a predilection to the trunk. No diagnostic changes are usually seen on histology. Elastic stains often reveal no abnormalities. OBJECTIVE: To review our cases of IAPP, describe their clinical and histological findings and compare them with the literature. METHODS: Retrospective review of IAPP cases who presented to our institution between 1994 and 2006. RESULTS: From a total of 16 patients, only 19% displayed hyperpigmented lesions, while 81% had either hypopigmented (9/16) or skin-colored (4/16) lesions. The sites predominantly affected were the lower extremities (62.5%), followed by the upper extremities and trunk. Only in one patient was IAPP co-existent with morphea. Histology revealed no diagnostic abnormalities; however, elastic stains showed a spectrum of changes ranging from normal to severe diminution and fragmentation of elastic fiber network. CONCLUSIONS: Our study shows several new aspects of IAPP. Clinically, the lesions were most commonly hypopigmented and involved predominantly the extremities. Histologically, IAPP exhibited a spectrum of alterations in elastic fibers. The most prevalent form of IAPP in our country seems to be unassociated with morphea.